Contents

Signs and symptoms

People with bronchiectasis produce frequent green/yellow sputum (what is distinctive is that patients with bronchiectasis can fill 240ml (8 oz) glasses with their daily sputum production). There may have bad breath and, when associated with cystic fibrosis, diarrhoea (because patients with bronchiectasis often develop chronic pancreatitis).

Diagnosis

The diagnosis of bronchiectasis is based on the review of clinical history and characteristic patterns in high-resolution CT scan findings. Such patterns include "tree-in-bud" abnormalities and cysts with definable borders. In one small study, CT findings of bronchiectasis and multiple small nodules were reported to have a sensitivity of 80%, specificity of 87%, and accuracy of 80% for the detection of bronchiectasis. Bronchiectasis may also be diagnosed without CT scan confirmation if clinical history clearly demonstrates frequent respiratory infections as well as confirmation of an underlying problem via blood work and sputum culture samples.[2]

Causes

Bronchiectasis, gross pathology.

Bronchiectasis secondary to a large carcinoid tumor (not shown) that was completely obstructing the bronchus proximally. The yellowish discoloration of lung parenchyma reflects obstructive pneumonia.

Acquired causes

Acquired Immune Deficiency Syndrome (AIDS) is the leading cause of bronchiectasis, especially in children. AIDS predisposes patients to a variety of pulmonary ailments, such as pneumonia and other opportunistic infections.[3]

Recent evidence has shown an increased risk of bronchiectasis in patients with rheumatoid arthritis who smoke. One study stated a tenfold increased prevalence of the disease in this cohort.[6] Still, it is unclear as to whether or not cigarette smoke is a specific primary cause of bronchiectasis.

Congenital causes

Kartagener syndrome, which affects the mobility of cilia in the lungs,[9] aids in the development of the disease. Another common genetic cause is cystic fibrosis, in which a small number of patients develop severe localized bronchiectasis.[10]Young's syndrome, which is clinically similar to cystic fibrosis, is thought to significantly contribute to the development of bronchiectasis. This is due to the occurrence of chronic, sinopulmonary infections.[11] Patients with alpha 1-antitrypsin deficiency have been found to be particularly susceptible to bronchiectasis, for unknown reasons.[12] Other less-common congenital causes include primary immunodeficiencies, due to the weakened or nonexistent immune system response to severe, recurrent infections that commonly affect the lung.[13] Several other congenital disorders can also lead to bronchiectasis, including Williams-Campbell syndrome and Marfan syndrome.[14]

Prevention

In order to prevent bronchiectasis, children should be immunized against measles, pertussis and other acute respiratory infections of childhood. While smoking has not been found to be a direct cause of bronchiectasis, it is certainly an irritant that all patients should avoid in order to prevent the development of infections (such as bronchitis) and further complications.[15]

Treatment

Treatment of bronchiectasis includes controlling infections and bronchial secretions, relieving airway obstructions, removal of affected portions of lung by surgical removal or artery embolization and preventing complications. This includes the prolonged usage of antibiotics to prevent detrimental infections,[17] as well as eliminating accumulated fluid with postural drainage and chest physiotherapy. Surgery may also be used to treat localized bronchiectasis, removing obstructions that could cause progression of the disease.[18]

Although not approved for use in any country, Mannitol dry inhalation powder, under the name Bronchitol, has been granted orphan drug status by the FDA for use in patients with bronchiectasis and with cystic fibrosis.[21]

Combination therapies, long acting bronchodilators and inhaled corticosteroids such as Symbicort and Advair Diskus are also commonly used inhaled medicines which has in many cases been effective in clearing the airways, reducing sputum and reducing inflammation.

History

Rene Laënnec, the man who invented the stethoscope, used his invention to first discover bronchiectasis in 1819.[22] The disease was researched in greater detail by Sir William Osler in the late 1800s; it is suspected that Osler actually died of complications from undiagnosed bronchiectasis.[23]